Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children

doi:10.1016/0887-8994(85)90027-X

Pediatric Neurology

Volume 1, Issue 5, September–October 1985, Pages 274-277

https://doi.org/10.1016/0887-8994(85)90027-X Get rights and content

Abstract

The prognosis of 27 patients with moyamoya disease was studied. The ages at onset ranged from 11 months to 4–11/12 years. Follow-up study was performed within 4 years from the onset in 13, 5 to 9 years in 5, and 10 to 15 years in 9. Transient ischemic attacks (TIA) occurred most often during the first four years and decreased thereafter. Intellectual deterioration and neurologic deficits increased with time. Outcome included no sequelae in five (19%), occasional TIA or headache alone in nine (33%), mild intellectual and/or motor impairment in seven (26%), requirement for special school or care by parents or institutions after reaching the teen years in three (11%), continuous 24-hour care in two (7%), and death in one (3%). Poor prognosis was correlated with an early age at onset and hypertension.

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Cited by (135)

Post-Surgical Cognitive Outcomes of Moyamoya Disease: A Systematic Review
2023, World Neurosurgery
Moyamoya disease (MMD) is an occlusive arteriopathy leading to stroke. Progressive if left untreated, revascularization surgery has become the mainstay of treatment. Although clinical and radiographic outcomes of MMD after intervention are well-characterized, cognitive outcomes in pediatric patients remain unclear. We aimed to examine postoperative cognitive outcomes in children with MMD, examine factors associated with cognitive changes after intervention, and define the effect of revascularization surgery on cognitive outcomes.
A systematic review was conducted following PRISMA guidelines searching PubMed, Embase, and Scopus databases. Articles met inclusion criteria if they studied pediatric patients undergoing revascularization surgery for MMD and examined cognitive outcomes either qualitatively or quantitatively. All data extracted from included articles was examined descriptively.
Of 1091 resultant articles, 12 articles containing 446 patients were included. Surgery was associated with maintained or improved full scale intellectual quotient (IQ), performance IQ, perceptual IQ, memory quotient and verbal memory. However, 70% of patients had impaired cognitive function, with associated poor school performance. Improvements in cognition were associated with increased cerebral blood flow, particularly to the middle cerebral artery, due to the development of collaterals. Female sex, shorter duration from symptom onset to surgery, and surgery after age 7 were predictive of cognitive improvement. Completed ischemic stroke prior to surgery was associated with poorer cognitive outcomes.
Although children with MMD have improved cognitive outcomes following revascularization overall, a distinct subset experience cognitive impairment. Consideration of patient-specific and treatment-related factors is important to enable proper risk stratification and inform management approaches.
Risk of Cerebrovascular Events Among Childhood and Adolescent Patients Receiving Cranial Radiation Therapy: A Pediatric Normal Tissue Effects in the Clinic Normal Tissue Outcomes Comprehensive Review
2022, International Journal of Radiation Oncology Biology Physics
Radiation-induced cerebrovascular toxicity is a well-documented sequelae that can be both life-altering and potentially fatal. We performed a meta-analysis of the relevant literature to create practical models for predicting the risk of cerebral vasculopathy after cranial irradiation.
A literature search was performed for studies reporting pediatric radiation therapy (RT) associated cerebral vasculopathy. When available, we used individual patient RT doses delivered to the Circle of Willis (CW) or optic chiasm (as a surrogate), as reported or digitized from original publications, to formulate a dose-response. A logistic fit and a Normal Tissue Complication Probability (NTCP) model was developed to predict future risk of cerebrovascular toxicity and stroke, respectively. This NTCP risk was assessed as a function of prescribed dose.
The search identified 766 abstracts, 5 of which were used for modeling. We identified 101 of 3989 pediatric patients who experienced at least one cerebrovascular toxicity: transient ischemic attack, stroke, moyamoya, or arteriopathy. For a range of shorter follow-ups, as specified in the original publications (approximate attained ages of 17 years), our logistic fit model predicted the incidence of any cerebrovascular toxicity as a function of dose to the CW, or surrogate structure: 0.2% at 30 Gy, 1.3% at 45 Gy, and 4.4% at 54 Gy. At an attained age of 35 years, our NTCP model predicted a stroke incidence of 0.9% to 1.3%, 1.8% to 2.7%, and 2.8% to 4.1%, respectively at prescribed doses of 30 Gy, 45 Gy, and 54 Gy (compared with a baseline risk of 0.2%-0.3%). At an attained age of 45 years, the predicted incidence of stroke was 2.1% to 4.2%, 4.5% to 8.6%, and 6.7% to 13.0%, respectively at prescribed doses of 30 Gy, 45 Gy, and 54 Gy (compared with a baseline risk of 0.5%-1.0%).
Risk of cerebrovascular toxicity continues to increase with longer follow-up. NTCP stroke predictions are very sensitive to model variables (baseline stroke risk and proportional stroke hazard), both of which found in the literature may be systematically erring on minimization of true risk. We hope this information will assist practitioners in counseling, screening, surveilling, and facilitating risk reduction of RT-related cerebrovascular late effects in this highly sensitive population.
Brain Compression by Encephalo-Myo-Synangiosis is a Risk Factor for Transient Neurological Deficits After Surgical Revascularization in Pediatric Patients with Moyamoya Disease
2020, World Neurosurgery
Citation Excerpt :
Pediatric moyamoya disease patients usually present with ischemic attacks and natural intellectual faculties that deteriorate over time.1
In pediatric patients with moyamoya disease, the pathophysiology of transient neurological deficits and the clinical features of perioperative cerebral blood flow (CBF) changes are unclear. The purpose of this study was to investigate the risk factors of postoperative transient neurological deficits and identify predictors of perioperative CBF changes.
This retrospective study included 42 surgical procedures in 28 pediatric patients who underwent surgical revascularization for moyamoya disease, including encephalo-myo-synangiosis (EMS) with or without superficial temporal artery–middle cerebral artery (MCA) anastomosis. Magnetic resonance images and single photon emission computed tomography results were obtained. Brain compression by EMS was also checked in fluid attenuated inversion recovery images. Using single photon emission computed tomography, CBF was measured at each anterior and posterior part of the MCA region.
Postoperative transient neurological deficits were observed in 12 (28.6%) out of 42 surgical procedures. Brain compression by EMS was a significant risk for transient neurological deficits (P = 0.009). The postoperative CBF in the anterior region increased in 9 cases (21.4%) and decreased in 10 cases (23.8%); in the posterior region, it increased in 12 cases (28.6%) and decreased in 10 cases (23.8%). Preoperative CBF of the anterior region was significantly related to both perioperative CBF changes in the MCA regions (anterior part, P = 0.004; posterior part, P = 0.025).
Brain compression by EMS is a risk factor for postoperative transient neurological deficits in pediatric patients with moyamoya disease, and preoperative CBF of the anterior MCA region could predict perioperative CBF change in the MCA regions.
Revascularization Surgery in Patients with Ischemic-Type Moyamoya Disease: Predictors for Postoperative Stroke and Long-Term Outcomes
2019, World Neurosurgery
Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD). This study aims to identify predictors for postoperative stroke and unfavorable outcome in ischemic-type MMD.
We identified a consecutive series of patients with ischemic-type MMD who underwent revascularization between January 2005 and December 2012. Predictors for postoperative stroke and functional outcomes were assessed with logistic and Cox regression analysis.
A total of 346 patients underwent 437 revascularization procedures and the mean follow-up period was 4.0 years. The incidence of perioperative stroke was 6.9%. Being adult at onset (odds ratio [OR], 5.033; 95% confidence interval [CI], 1.447–17.506; P = 0.011) and posterior cerebral artery (PCA) stenosis (OR, 3.364; 95% CI, 1.588–7.265; P = 0.002) before surgery were predictors of perioperative stroke. The annual subsequent stroke rate beyond 30 days after surgery was 1.2%. Subsequent stroke events tended to occur throughout the first 5 years after surgery in adults, whereas in children they mainly occurred within the first 2 years after surgery. Age at onset (OR, 1.025; 95% CI, 1.003–1.048; P = 0.023), ischemic stroke or transient ischemic attack at presentation (OR, 2.703; 95% CI, 1.062–6.875; P = 0.037), and PCA involvement (OR, 2.664; 95% CI, 1.462–4.854; P = 0.001) were associated with higher risk of overall postoperative stroke. PCA involvement (OR, 2.62; 95% CI, 1.33–5.15; P = 0.005), internal carotid artery supraclinoid segment occlusion (OR, 2.76; 95% CI, 1.27–6.03; P = 0.011), and older age at onset (OR, 1.03; 95% CI, 1.01–1.05; P = 0.033) were predictive of unfavorable outcome.
Patients with ischemic-type MMD at an older age and more severe angiopathy might be at higher risk of recurrent stroke and unfavorable outcome after revascularization.
French clinical practice guidelines for Moyamoya angiopathy
2018, Revue Neurologique
Citation Excerpt :
Only few comparative, non-randomized and mostly retrospective studies with a limited number of patients are available. These studies do not provide an answer about the superiority of surgical treatment [10–16,41,42,49]. Several series on a large number of operated patients demonstrate a significant reduction of ischemic cerebral events after direct or indirect surgical revascularization in the adult and pediatric forms of MMA suggesting some efficacy of this treatment [17,18,41–46,49].
Neuropsychiatric Symptoms of Moyamoya Disease: Considerations for the Clinician
2024, Neuropsychiatric Disease and Treatment

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^☆: The study was funded by The Committee on Research of Spontaneous Occlusion of the Circle of Willis (“Moyamoya” disease) of the Ministry of Health and Welfare, Japan.

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Original articlePrognosis of occlusive disease of the circle of Willis (moyamoya disease) in children☆

Abstract

Brain Develop

Pediatr Clin North Am

Human Pathol

Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain

Arch Neurol

Hypoplasia and obstruction of the circle of Willis in a case of atypical cerebral hemorrhage and its relationship to Nishimoto's disease

Neurology

Familial occurrence of moyamoya disease: report of three Japanese families

J Neurol Neurosurg Psychiatry

Abnormal cerebrovascular network related to the internal carotid arteries

J Neurosurg

Spontaneous occlusion of the circle of Willis

Neurology

Cerebrovascular occlusive disease with and without the moyamoya vascular network in children

Neuropediatrics

Electroencephalographic findings in children with moyamoya disease

Arch Neurol

Computed tomography in “moyamoya”: Summary of 16 cases

No To Shinkei

Computed tomography of moyamoya disease: demonstration of occluded arteries and collateral vessels as important diagnostic signs

Radiology

Original article
Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children☆